Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen Journal Article


Authors: Jaeckle, K. A.; Graus, F.; Houghton, A.; Cardon‐Cardo, C.; Nielsen, S. L.; Posner, J. B.
Article Title: Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen
Abstract: Sera from 6 of 12 patients with paraneoplastic cerebellar degeneration (PCD) contained anti–Purkinje cell antibodies, as determined by indirect immunofluorescence on frozen sections of normal human cerebellum. Samples of cerebrospinal fluid from 2 of the patients with serum antibodies were tested, and both specimens contained anti–Purkinje cell antibody. The anti‐Purkinje cell antibodies were polyclonal, fixed complement, and were present in all patients at serum dilutions of 1:1,000 or greater. Antibody activity could not be suppressed by preabsorption of sera with human or animal brain and tissue powders or with fresh crude human cerebellar extracts. No anti–Purkinje cell antibodies were detected in control sera from 167 neurologically normal cancer patients, 32 normal volunteers, 10 patients with other causes of cerebellar degeneration, or 8 patients with other paraneoplastic neurological diseases. Preliminary evidence suggests that the Purkinje antigen is a protein that is often concentrated in the periphery of the cytoplasm in disc‐shaped structures. Patients with antibodies often developed signs of PCD near the time of detection of the tumor and had relentless progression of neurological disease. Patients without antibodies frequently had cancer for months to years before PCD developed, and often had spontaneous stabilization of neurological disease with time. Four patients without and 3 patients with antibodies underwent plasmapheresis without response. Copyright © 1985 American Neurological Association
Keywords: aged; middle aged; major clinical study; neoplasms; cerebellum; purkinje cell; purkinje cells; immunofluorescence; central nervous system; immunoenzyme techniques; fluorescent antibody technique; antigens; cytoplasm; autoantigens; autoimmunity; paraneoplastic syndrome; autoimmune diseases; autoantibodies; nerve degeneration; female genital system; epitopes; etiology; cerebellar diseases; respiratory system; paraneoplastic syndromes; cytoplasm protein; humans; human; priority journal
Journal Title: Annals of Neurology
Volume: 18
Issue: 5
ISSN: 0364-5134
Publisher: Wiley Blackwell  
Date Published: 1985-11-01
Start Page: 592
End Page: 600
Language: English
DOI: 10.1002/ana.410180513
PROVIDER: scopus
PUBMED: 2416270
DOI/URL:
Notes: Article -- Export Date: 26 October 2021 -- Source: Scopus
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